The effect of hypertension on cardiac structure and function in different types of hypertrophic cardiomyopathy: A single-center retrospective study

Objectives: To determine whether hypertension (HTN) affects cardiac structure and function in different types of hypertrophic cardiomyopathy (HCM).

Design: Patients with obstructive HCM (n = 40), septal HCM (n = 88), and apical HCM (n = 42) were separated into hypertensive and non-hypertensive subgroups, and echocardiographic parameters at baseline and at follow-up were compared between the subgroups.

Results: At follow-up, hypertensive obstructive HCM patients showed a decrease in end-diastolic volume (from 93.87 ± 26.08 mL to 79.06 ± 20.07 mL; p= 0.045) and in left ventricular end-diastolic diameter (from 45.00 ± 5.32 mm to 41.83 ± 4.58 mm; p =0.042). Non-hypertensive obstructive HCM patients showed a decrease in maximum aortic velocity (from 2.01 ± 0.53 m/s to 1.28 ± 0.25 m/s; p= 0.011) and in aortic maximum pressure gradient (from 17.22 ± 9.57 mm Hg to 6.79 ± 2.44 mm Hg; p= 0.03). Hypertensive apical HCM patients showed an increase in end-diastolic volume (from 95.28 ± 16.54 mL to 119.74 ± 25.19 mL; p= 0.016) and in left ventricular end-diastolic diameter (from 45.28 ± 3.36 mm to 50.20 ± 4.56 mm; p= 0.007).

Conclusions: HTN can affect left ventricular capacity in obstructive HCM and apical HCM, causing a decrease in ventricular capacity in the former and increase in the latter; it has no significant effect on the size of the left ventricular cavity in septal HCM. HTN can lead to a poor therapeutic effect on aortic flow rate and pressure gradient in obstructive HCM patients.     

Association between Parity,Carotid Plaques,and Intima Media Thickness in Northern Chinese Women

Pregnancy is always associated with several important physiological changes in women, including sex hormone levels, glycolipid metabolism, and oxidative stress. These changes may have short-term and long-term effects on their cardiovascular system. Parity is the number of times a woman has given birth. Several studies have investigated the association between parity and risk of cardiovascular diseases in women. Prospective studies have shown a fairly low incidence of cardiovascular endpoints for parous women[1]. Therefore, it would be meaningful to assess the relationship between parity and other surrogate markers.     

新的珠蛋白基因突变引起少见地中海贫血四例报告及文献复习

目的分析4例少见地中海贫血（地贫）患者的DNA序列、临床表型，提高对地贫的认识。方法对2014年5月至2019年12月4例少见地贫患者的临床及DNA序列特征进行回顾性分析并复习相关文献。结果地贫基因常规检测显示，例1～3均未检测到常见的3种α株蛋白1/2（HBA1/A2）基因缺失及其3种点突变和16种β株蛋白（HBB）基因点突变，例4检测到αα^--SEA缺失。HBA1/A2和HBB基因全序列Sanger测序示：例1～4分别存在HBB:c.347C>A、HBB:c.1A>G、HBB:c.393T>G及HBA2:c.301-1G>A（IVS-II-142 G>A）突变。同时，例2的祖父、父亲和姑姑均为HBB:c.1A>G杂合突变。结论本研究发现了新的珠蛋白基因突变，HBB:c.347C>A、HBB:c.1A>G和HBB:c.393T>G以及HBA2:c.301-1 G>A（IVS-II-142 G>A）突变在中国地贫患者中为首次报道，HBB:c.393T>G突变为全球首次报道，丰富了地贫基因突变数据库。     

Multicenter randomized study of inosine pranobex versus acyclovir in the treatment of recurrent herpes labialis and recurrent herpes genitalis in Chinese patients

The objective of the study is to evaluate the efficacy and safety of oral inosine pranobex as compared with acyclovir in the treatment of recurrent herpes labialis (RHL) and recurrent herpes genitalis (RHG). A multicenter double‐blind, double‐dummy, randomized, controlled, parallel group trial was conducted in 144 patients with RHL and 144 RHG. Patients were assigned to treatment in one of two groups: (i) inosine pranobex group (active inosine pranobex, 1 g four times daily, and acyclovir placebo); or (ii) acyclovir group (active acyclovir, 200 mg five times daily, and inosine pranobex placebo). The total symptom score (TSS) of patients with RHL did not differ in the inosine pranobex and acyclovir group on the 3rd or 7th day of treatment. There was also no difference in the efficacy rates between the two groups. No difference of TSS was observed between patients with RHG taking inosine pranobex and acyclovir on days 3 or 5 of the treatment, respectively. The short‐term clinical recurrence rate of RHG at 3‐month follow‐up was much lower in the inosine pranobex group than acyclovir group. The incidence of hyperuricemia was higher in the inosine pranobex group than acyclovir group. In conclusion, inosine pranobex was as effective as acyclovir in treating RHL and RHG with significantly greater reduction of the short‐term recurrence rate of herpes genitalis at 3‐month follow up. Long‐term recurrence rates at 6 months or longer remain to be determined. Hyperuricemia should be monitored during the treatment.     

Survival prediction for gallbladder carcinoma after curative resection: Comparison of nomogram and Bayesian network models

BackgroundIn this study, we developed a nomogram and a Bayesian network (BN) model for prediction of survival in gallbladder carcinoma (GBC) patients following surgery and compared the performance of the two models.MethodsSurvival prediction models were established and validated using data from 698 patients with GBC who underwent curative-intent resection between 2008 and 2017 at one of six Chinese tertiary hospitals. Model construction and internal validation were performed using data from 381 patients at one hepatobiliary center, and external validation was then performed using data from 317 patients at the other five centers. A BN model and a nomogram model were constructed based on the independent prognostic variables. Performance of the BN and nomogram models was compared based on area under receiver operating characteristic curves (AUC), model accuracy, and a confusion matrix.ResultsIndependent prognostic variables included age, pathological grade, liver infiltration, T stage, N stage, and margin. In internal validation, AUC was 84.14% and 78.22% for the BN and nomogram, respectively, and model accuracy was 75.65% and 72.17%, respectively. In external validation, AUC was 76.46% and 70.19% for the BN and nomogram, respectively, with model accuracy of 66.88% and 60.25%, respectively. Based on the confusion matrix, the nomogram had a higher true positive rate but a substantially lower true negative rate compared to the BN.ConclusionA BN model was more accurate than a Cox regression-based nomogram for prediction of survival in GBC patients undergoing curative-intent resection.     

Atomoxetine,a norepinephrine reuptake inhibitor,reduces seizure-induced respiratory arrest

Sudden unexpected death in epilepsy (SUDEP) is a devastating epilepsy complication, and no effective preventive strategies are currently available for this fatal disorder. Clinical and animal studies of SUDEP demonstrate that seizure-induced respiratory arrest (S-IRA) is the primary event leading to death after generalized seizures in many cases. Enhancing brain levels of serotonin reduces S-IRA in animal models relevant to SUDEP, including the DBA/1 mouse. Given that serotonin in the brain plays an important role in modulating respiration and arousal, these findings suggest that deficits in respiration and/or arousal may contribute to S-IRA. It is well known that norepinephrine is an important neurotransmitter that modulates respiration and arousal in the brain as well. Therefore, we hypothesized that enhancing noradrenergic neurotransmission suppresses S-IRA. To test this hypothesis, we examined the effect of atomoxetine, a norepinephrine reuptake inhibitor (NRI), on S-IRA evoked by either acoustic stimulation or pentylenetetrazole in DBA/1 mice. We report the original observation that atomoxetine specifically suppresses S-IRA without altering the susceptibility to seizures evoked by acoustic stimulation, and atomoxetine also reduces S-IRA evoked by pentylenetetrazole in DBA/1 mice. Our data suggest that the noradrenergic signaling is importantly involved in S-IRA, and that atomoxetine, a medication widely used to treat attention deficit hyperactivity disorder (ADHD), is potentially useful to prevent SUDEP.     

Real-world treatment patterns and outcomes of pyrotinib-based therapy in patients with HER2-positive advanced breast cancer (PRETTY): A nationwide,prospective, observational study

Pyrotinib, an irreversible pan-ErbB inhibitor, has been approved for treating HER2-positive advanced breast cancer in China. We conducted a nationwide, prospective observational study to examine the real-world data of pyrotinib-based therapy in this population. Patients from 61 sites across China were included. Pyrotinib-based regimens were prescribed at local physician's discretion. Demographics, treatment patterns, prognosis and safety were evaluated. The primary outcome was real-world progression-free survival (rwPFS). Of 1129 patients, pyrotinib-based therapy was prescribed as first-, second- and third- or later-line treatment in 437 (38.7%), 476 (42.2%) and 216 (19.1%) patients, respectively. Median rwPFS (mrwPFS) was 14.3 (95% CI, 13.3-15.2) months in the total population, with the longest mrwPFS of 17.8 (95% CI, 15.2-24.9) months in the first-line setting, followed by 14.4 (95% CI, 12.9-15.3) months in the second-line setting. Patients with third- or later-line treatment also achieved a mrwPFS of 9.3 (95% CI, 8.4-11.8) months. Patients with trastuzumab- or trastuzumab-pertuzumab-treated disease achieved a mrwPFS of 14.3 and 13.6 months, respectively. Dual HER2 blockade with pyrotinib plus trastuzumab showed a mrwPFS of 16.2 months in the total population, with data not mature in the first-line setting. For patients with baseline brain metastases, the mrwPFS was 11.7 months. The most common adverse event was diarrhea (any grade, 73.5%; grade ≥ 3, 15.3%). In real world, pyrotinib-based therapy shows promising effectiveness in the first-, as well as second- and later-line treatment, with acceptable tolerability. Further investigations regarding front-line use or novel combinations of pyrotinib might facilitate to maximize its anti-tumor potential.     

FⅦ活化蛋白酶基因的Marburg Ⅰ型多态性与脑梗死发病的相关性

目的：探讨FⅦ活化蛋白酶（Factor Ⅶ-activating protease,FSAP）基因的Marburg Ⅰ型多态性与脑梗死发病之间的相关性,并分析FSAP的Marburg I型多态性是否为脑梗死的危险因子之一。方法：应用单链构象多态性PCR技术（SSCP-PCR）,对159例临床确诊的脑梗死患者及179例年龄、性别相匹配的无血栓性疾病病史志愿者的FSAP基因进行多态性分析。结果：FSAP基因表型在病例组及对照组中均为野生型纯合子,未检测到Marburg Ⅰ型突变型。发现1例FSAP基因点突变（C1815T）。结论：FSAP基因的Marburg Ⅰ型多态性与脑梗死发病可能无相关性。